Six patients with neurological complications in Rendu-Osler-Weber disease were reviewed. There were 4 patients (3 int the same family) with a brain abscess, one patient with a brain abscess and a spinal cord subarachnoid hemorrhage, one patient with a recurrent intracerebral hemorrhage due to a vascular malformation. The infectious complications are related to the presence of pulmonary arterio-venous fistula, which should be treated, whenever diagnosed. Because of the potentially severe outcome of these neurological complications of the Rendu-Osler-Weber disease, screening of the patient's relatives is highly recommended.